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Protein

Complement component C8 beta chain

Gene

C8B

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells.

GO - Biological processi

  • complement activation Source: ProtInc
  • complement activation, alternative pathway Source: UniProtKB-KW
  • complement activation, classical pathway Source: UniProtKB-KW
  • cytolysis Source: UniProtKB-KW
  • immune response Source: ProtInc
  • regulation of complement activation Source: Reactome
Complete GO annotation...

Keywords - Biological processi

Complement alternate pathway, Complement pathway, Cytolysis, Immunity, Innate immunity

Enzyme and pathway databases

ReactomeiR-HSA-166665. Terminal pathway of complement.
R-HSA-977606. Regulation of Complement cascade.

Names & Taxonomyi

Protein namesi
Recommended name:
Complement component C8 beta chain
Alternative name(s):
Complement component 8 subunit beta
Gene namesi
Name:C8B
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:1353. C8B.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • extracellular region Source: Reactome
  • extracellular space Source: Ensembl
  • extracellular vesicle Source: UniProtKB
  • membrane Source: ProtInc
  • membrane attack complex Source: UniProtKB-KW
Complete GO annotation...

Keywords - Cellular componenti

Membrane attack complex, Secreted

Pathology & Biotechi

Involvement in diseasei

Complement component 8 deficiency, 2 (C8D2)
Disease susceptibility is associated with variations affecting the gene represented in this entry.
Disease descriptionA rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.
See also OMIM:613789

Organism-specific databases

MalaCardsiC8B.
MIMi613789. phenotype.
Orphaneti169150. Immunodeficiency due to a late component of complements deficiency.
PharmGKBiPA25952.

Polymorphism and mutation databases

DMDMi20141201.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 3232Sequence analysisAdd
BLAST
Propeptidei33 – 54221 PublicationPRO_0000023591Add
BLAST
Chaini55 – 591537Complement component C8 beta chainPRO_0000023592Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Disulfide bondi65 ↔ 1001 Publication
Glycosylationi70 – 701C-linked (Man)1 Publication
Glycosylationi73 – 731C-linked (Man)1 Publication
Disulfide bondi76 ↔ 1101 Publication
Disulfide bondi79 ↔ 1161 Publication
Glycosylationi101 – 1011N-linked (GlcNAc...)Sequence analysis
Disulfide bondi122 ↔ 1331 Publication
Disulfide bondi127 ↔ 1461 Publication
Disulfide bondi140 ↔ 1551 Publication
Glycosylationi243 – 2431N-linked (GlcNAc...)2 Publications
Disulfide bondi378 ↔ 4031 Publication
Modified residuei418 – 4181Phosphothreonine1 Publication
Disulfide bondi503 ↔ 5501 Publication
Disulfide bondi505 ↔ 5211 Publication
Disulfide bondi508 ↔ 5231 Publication
Disulfide bondi525 ↔ 5341 Publication
Glycosylationi551 – 5511C-linked (Man)1 Publication
Glycosylationi554 – 5541C-linked (Man)1 Publication
Disulfide bondi557 ↔ 5901 Publication

Post-translational modificationi

N-glycosylated; contains one or two bound glycans. Not O-glycosylated.4 Publications

Keywords - PTMi

Disulfide bond, Glycoprotein, Phosphoprotein

Proteomic databases

PaxDbiP07358.
PeptideAtlasiP07358.
PRIDEiP07358.

PTM databases

iPTMnetiP07358.
PhosphoSiteiP07358.

Expressioni

Gene expression databases

BgeeiENSG00000021852.
CleanExiHS_C8B.
ExpressionAtlasiP07358. baseline and differential.
GenevisibleiP07358. HS.

Organism-specific databases

HPAiHPA023694.

Interactioni

Subunit structurei

Heterotrimer of 3 chains: alpha, beta and gamma. The alpha and gamma chains are disulfide bonded. Component of the membrane attack complex (MAC). MAC assembly is initiated by protelytic cleavage of C5 into C5a and C5b. C5b binds sequentially C6, C7, C8 and multiple copies of the pore-forming subunit C9.2 Publications

Protein-protein interaction databases

BioGridi107193. 2 interactions.
STRINGi9606.ENSP00000360281.

Structurei

Secondary structure

1
591
Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Turni78 – 814Combined sources
Beta strandi82 – 865Combined sources
Beta strandi89 – 913Combined sources
Beta strandi104 – 1096Combined sources
Beta strandi123 – 1264Combined sources
Turni128 – 1303Combined sources
Helixi136 – 1383Combined sources
Beta strandi141 – 1433Combined sources
Beta strandi146 – 1494Combined sources
Helixi172 – 1765Combined sources
Beta strandi177 – 1793Combined sources
Turni181 – 1833Combined sources
Beta strandi186 – 1894Combined sources
Beta strandi201 – 2055Combined sources
Beta strandi208 – 2125Combined sources
Beta strandi216 – 2216Combined sources
Beta strandi229 – 2368Combined sources
Helixi237 – 2404Combined sources
Turni256 – 2583Combined sources
Turni269 – 2713Combined sources
Beta strandi272 – 2754Combined sources
Helixi276 – 2838Combined sources
Beta strandi290 – 2978Combined sources
Beta strandi299 – 3068Combined sources
Beta strandi308 – 3103Combined sources
Helixi315 – 3217Combined sources
Helixi330 – 34011Combined sources
Beta strandi342 – 35211Combined sources
Beta strandi355 – 3617Combined sources
Helixi362 – 3654Combined sources
Turni366 – 3694Combined sources
Helixi372 – 3787Combined sources
Turni379 – 3813Combined sources
Turni404 – 4085Combined sources
Helixi409 – 4124Combined sources
Turni413 – 4153Combined sources
Beta strandi419 – 4268Combined sources
Helixi435 – 4395Combined sources
Beta strandi441 – 4433Combined sources
Helixi449 – 4579Combined sources
Beta strandi461 – 4699Combined sources
Helixi470 – 4734Combined sources
Turni476 – 4783Combined sources
Helixi482 – 49918Combined sources
Helixi502 – 5043Combined sources
Beta strandi513 – 5175Combined sources
Beta strandi520 – 5245Combined sources
Helixi532 – 5343Combined sources
Beta strandi536 – 5416Combined sources
Beta strandi557 – 5637Combined sources
Beta strandi584 – 5885Combined sources

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
3OJYX-ray2.51B55-591[»]
ProteinModelPortaliP07358.
SMRiP07358. Positions 61-590.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP07358.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini64 – 11754TSP type-1 1PROSITE-ProRule annotationAdd
BLAST
Domaini120 – 15738LDL-receptor class APROSITE-ProRule annotationAdd
BLAST
Domaini158 – 504347MACPFPROSITE-ProRule annotationAdd
BLAST
Domaini505 – 53531EGF-likeAdd
BLAST
Domaini545 – 59147TSP type-1 2PROSITE-ProRule annotationAdd
BLAST

Sequence similaritiesi

Belongs to the complement C6/C7/C8/C9 family.Curated
Contains 1 EGF-like domain.Curated
Contains 1 LDL-receptor class A domain.PROSITE-ProRule annotation
Contains 1 MACPF domain.PROSITE-ProRule annotation
Contains 2 TSP type-1 domains.PROSITE-ProRule annotation

Keywords - Domaini

EGF-like domain, Repeat, Signal

Phylogenomic databases

eggNOGiENOG410IE7H. Eukaryota.
ENOG410Y2J1. LUCA.
HOGENOMiHOG000231146.
HOVERGENiHBG106489.
InParanoidiP07358.
KOiK03998.
OrthoDBiEOG091G07LK.
PhylomeDBiP07358.
TreeFamiTF330498.

Family and domain databases

InterProiIPR023415. LDLR_class-A_CS.
IPR002172. LDrepeatLR_classA_rpt.
IPR001862. MAC_perforin.
IPR020864. MACPF.
IPR020863. MACPF_CS.
IPR000884. TSP1_rpt.
[Graphical view]
PfamiPF00057. Ldl_recept_a. 1 hit.
PF01823. MACPF. 1 hit.
PF00090. TSP_1. 2 hits.
[Graphical view]
PRINTSiPR00764. COMPLEMENTC9.
SMARTiSM00192. LDLa. 1 hit.
SM00457. MACPF. 1 hit.
SM00209. TSP1. 2 hits.
[Graphical view]
SUPFAMiSSF57424. SSF57424. 1 hit.
SSF82895. SSF82895. 2 hits.
PROSITEiPS00022. EGF_1. 1 hit.
PS01209. LDLRA_1. 1 hit.
PS50068. LDLRA_2. 1 hit.
PS00279. MACPF_1. 1 hit.
PS51412. MACPF_2. 1 hit.
PS50092. TSP1. 2 hits.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P07358-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MKNSRTWAWR APVELFLLCA ALGCLSLPGS RGERPHSFGS NAVNKSFAKS
60 70 80 90 100
RQMRSVDVTL MPIDCELSSW SSWTTCDPCQ KKRYRYAYLL QPSQFHGEPC
110 120 130 140 150
NFSDKEVEDC VTNRPCRSQV RCEGFVCAQT GRCVNRRLLC NGDNDCGDQS
160 170 180 190 200
DEANCRRIYK KCQHEMDQYW GIGSLASGIN LFTNSFEGPV LDHRYYAGGC
210 220 230 240 250
SPHYILNTRF RKPYNVESYT PQTQGKYEFI LKEYESYSDF ERNVTEKMAS
260 270 280 290 300
KSGFSFGFKI PGIFELGISS QSDRGKHYIR RTKRFSHTKS VFLHARSDLE
310 320 330 340 350
VAHYKLKPRS LMLHYEFLQR VKRLPLEYSY GEYRDLFRDF GTHYITEAVL
360 370 380 390 400
GGIYEYTLVM NKEAMERGDY TLNNVHACAK NDFKIGGAIE EVYVSLGVSV
410 420 430 440 450
GKCRGILNEI KDRNKRDTMV EDLVVLVRGG ASEHITTLAY QELPTADLMQ
460 470 480 490 500
EWGDAVQYNP AIIKVKVEPL YELVTATDFA YSSTVRQNMK QALEEFQKEV
510 520 530 540 550
SSCHCAPCQG NGVPVLKGSR CDCICPVGSQ GLACEVSYRK NTPIDGKWNC
560 570 580 590
WSNWSSCSGR RKTRQRQCNN PPPQNGGSPC SGPASETLDC S
Length:591
Mass (Da):67,047
Last modified:January 23, 2002 - v3
Checksum:iB01722A6F2E9AFCE
GO

Polymorphismi

The sequence shown is that of allotype C8B B.

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti108 – 1081E → K.
Corresponds to variant rs12067507 [ dbSNP | Ensembl ].
VAR_027649
Natural varianti117 – 1171R → G in allotype C8B A. 2 Publications
Corresponds to variant rs1013579 [ dbSNP | Ensembl ].
VAR_012642
Natural varianti261 – 2611P → L.
Corresponds to variant rs12085435 [ dbSNP | Ensembl ].
VAR_027650

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M16973 mRNA. Translation: AAA51862.1.
AK313382 mRNA. Translation: BAG36180.1.
AL121998 Genomic DNA. Translation: CAC18532.1.
CH471059 Genomic DNA. Translation: EAX06641.1.
BC130575 mRNA. Translation: AAI30576.1.
X04393 mRNA. Translation: CAA27981.1.
CCDSiCCDS30730.1.
PIRiA43071. C8HUB.
RefSeqiNP_000057.2. NM_000066.3.
UniGeneiHs.391835.

Genome annotation databases

EnsembliENST00000371237; ENSP00000360281; ENSG00000021852.
GeneIDi732.
KEGGihsa:732.
UCSCiuc001cyp.5. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Web resourcesi

C8Bbase

C8B mutation db

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M16973 mRNA. Translation: AAA51862.1.
AK313382 mRNA. Translation: BAG36180.1.
AL121998 Genomic DNA. Translation: CAC18532.1.
CH471059 Genomic DNA. Translation: EAX06641.1.
BC130575 mRNA. Translation: AAI30576.1.
X04393 mRNA. Translation: CAA27981.1.
CCDSiCCDS30730.1.
PIRiA43071. C8HUB.
RefSeqiNP_000057.2. NM_000066.3.
UniGeneiHs.391835.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
3OJYX-ray2.51B55-591[»]
ProteinModelPortaliP07358.
SMRiP07358. Positions 61-590.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107193. 2 interactions.
STRINGi9606.ENSP00000360281.

PTM databases

iPTMnetiP07358.
PhosphoSiteiP07358.

Polymorphism and mutation databases

DMDMi20141201.

Proteomic databases

PaxDbiP07358.
PeptideAtlasiP07358.
PRIDEiP07358.

Protocols and materials databases

DNASUi732.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000371237; ENSP00000360281; ENSG00000021852.
GeneIDi732.
KEGGihsa:732.
UCSCiuc001cyp.5. human.

Organism-specific databases

CTDi732.
GeneCardsiC8B.
HGNCiHGNC:1353. C8B.
HPAiHPA023694.
MalaCardsiC8B.
MIMi120960. gene.
613789. phenotype.
neXtProtiNX_P07358.
Orphaneti169150. Immunodeficiency due to a late component of complements deficiency.
PharmGKBiPA25952.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IE7H. Eukaryota.
ENOG410Y2J1. LUCA.
HOGENOMiHOG000231146.
HOVERGENiHBG106489.
InParanoidiP07358.
KOiK03998.
OrthoDBiEOG091G07LK.
PhylomeDBiP07358.
TreeFamiTF330498.

Enzyme and pathway databases

ReactomeiR-HSA-166665. Terminal pathway of complement.
R-HSA-977606. Regulation of Complement cascade.

Miscellaneous databases

EvolutionaryTraceiP07358.
GenomeRNAii732.
PROiP07358.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000021852.
CleanExiHS_C8B.
ExpressionAtlasiP07358. baseline and differential.
GenevisibleiP07358. HS.

Family and domain databases

InterProiIPR023415. LDLR_class-A_CS.
IPR002172. LDrepeatLR_classA_rpt.
IPR001862. MAC_perforin.
IPR020864. MACPF.
IPR020863. MACPF_CS.
IPR000884. TSP1_rpt.
[Graphical view]
PfamiPF00057. Ldl_recept_a. 1 hit.
PF01823. MACPF. 1 hit.
PF00090. TSP_1. 2 hits.
[Graphical view]
PRINTSiPR00764. COMPLEMENTC9.
SMARTiSM00192. LDLa. 1 hit.
SM00457. MACPF. 1 hit.
SM00209. TSP1. 2 hits.
[Graphical view]
SUPFAMiSSF57424. SSF57424. 1 hit.
SSF82895. SSF82895. 2 hits.
PROSITEiPS00022. EGF_1. 1 hit.
PS01209. LDLRA_1. 1 hit.
PS50068. LDLRA_2. 1 hit.
PS00279. MACPF_1. 1 hit.
PS51412. MACPF_2. 1 hit.
PS50092. TSP1. 2 hits.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiCO8B_HUMAN
AccessioniPrimary (citable) accession number: P07358
Secondary accession number(s): A1L4K7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: April 1, 1988
Last sequence update: January 23, 2002
Last modified: September 7, 2016
This is version 167 of the entry and version 3 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.